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[Minimal pulmonary regurgitation (pulsed Doppler echocardiography findings) in the absence of pulmonary valve defect].

A diastolic turbulent flow of pulmonary regurgitation with the maximum velocity of no more than 1.5 m/sec and prevalence in the exit portion of the right ventricle up to 17 mm was detected in 36 patients with cardiac and pulmonary diseases and apparently healthy individuals without organic pulmonary valve lesion and diastolic localization at the respective site, by using pulsed Doppler echocardiography. The highest diastolic transpulmonary pressure gradient estimated by a modified Bernulli's equation was no more than 9 mm Hg (within the range of normal values for diastolic pulmonary artery-right ventricle pressure gradient). Asymptomatic subclinical pulmonary regurgitation of physiological and functional nature without substantial hemodynamic significance which has been detected in these patients cannot be regarded as a manifestation of pulmonary valve defect.

Adult↗

Allograft degeneration in infant pulmonary valve allograft recipients.

Infants who have undergone pulmonary valve allograft reconstruction of their right ventricular outflow tract (RVOT), experience an increased incidence of allograft fibrocalcification and valvar insufficiency, compared to older children. Since 1985, 137 pediatric patients have received a cryopreserved pulmonary valve allograft to repair their RVOT. One hundred and five patients were 1 year of age or more and 32 were younger than 1 year at operation. In the former group, there were 11 hospital deaths (10%). Ninety-four operative survivors have been followed clinically for one month to 7.2 years (mean: 3.4 years). One patient required cardiac transplant secondary to myocardial dysfunction. There have been three late deaths (3%), and two children (2%) have undergone reoperation to replace the allograft. In the infant group, there were eight hospital deaths (25%). During 1 month to 6.8 years (mean: 2.2 years) of clinical follow up, there have been six late deaths (25%), and four children (22%) have required allograft explant. The most common indication for reoperation has been allograft fibrocalcification and valvar insufficiency. The relatively high incidence of early allograft failure in infants, which may have an immunologic etiology, has prompted consideration of nonviable allografts or low dose cyclosporine as alternatives when surgical reconstruction of the RVOT is required in children younger than 1 year of age.

Adolescent↗

Regurgitation through the morphologically pulmonary valve after additional aortopulmonary anastomosis.

Although additional aortopulmonary anastomosis established by creating double outlet from the systemic ventricle and anastomosing the pulmonary trunk to the ascending aorta can undoubtedly provide an unobstructed outlet for the systemic ventricle, postoperative regurgitation of the morphologically pulmonary valve, if it occurs, may spoil the efficacy. To determine whether the pulmonary valve can be regurgitant, 11 patients undergoing this surgical option, six accompanying a Fontan-type operation and five associated with biventricular repair, were reviewed. Postoperative regurgitation was observed echocardiographically in five patients. One patient with severe pathologic changes of the valve showed progressive regurgitation, necessitating valve replacement two months after the aortopulmonary anastomosis. The other four patients, including one with slight thickening of the pulmonary leaflets, had previously undergone banding of the pulmonary trunk. Slight regurgitation in these cases appeared between one month and two years after the procedure, but then neither progressed nor regressed. Pathologic changes of the pulmonary valve, and previous banding, therefore, can be recognized as risk factors for postoperative regurgitation. The morphologically pulmonary valve, nonetheless, proved to be feasible for this surgical option unless it possessed severe organic changes.

Anastomosis, Surgical↗

Significance of pulmonary valve prolapse. A cross sectional echocardiographic study.

Many patients with congenital heart disease now undergo cardiac surgery based solely on clinical and echocardiographic findings, but those with intracardiac shunts still frequently require cardiac catheterisation because there is no reliable non-invasive method of measuring the pulmonary artery pressure. Blinded to the haemodynamic results two independent observers retrospectively studied the cross sectional echocardiograms of 59 patients with uncomplicated ventricular septal defect to assess whether diastolic backward bowing of the pulmonary valve leaflets towards the right ventricular outflow tract (pulmonary valve prolapse) was associated with pulmonary hypertension. There was considerable interobserver variation in the diagnosis of pulmonary valve prolapse, but concordance was achieved in 27 cases. Mean pulmonary artery systolic and mean and diastolic pressures and the ratios of aortic to pulmonary artery mean pressures were all significantly higher for the group with pulmonary valve prolapse diagnosed by both observers than for the group without, thus showing an association between pulmonary valve prolapse and pulmonary hypertension. Further studies are warranted to determine the usefulness of this cross sectional echocardiographic sign in routine clinical practice.

Aorta↗

Optimal timing for pulmonary valve replacement in adults after tetralogy of Fallot repair.

The timing of pulmonary valve replacement in adult patients with repaired tetralogy of Fallot remains controversial. A magnetic resonance imaging study in 17 adult patients with repaired tetralogy of Fallot reveals a statistically significant decrease in right ventricular (RV) volume (RV end-diastolic volume 163 +/- 34 to 107 +/- 26 ml/m2, p <0.001; RV end-systolic volume 109 +/- 27 to 69 +/- 22 ml/m2, p <0.001) at a mean follow-up of 21 months after pulmonary valve replacement; whereas RV systolic function remained unchanged (mean RV ejection fraction 32 +/- 7% to 34 +/- 10%, p = 0.12). In no patients with a RV end-diastolic volume >170 ml/m2 or a RV end-systolic volume >85 ml/m2 before pulmonary valve replacement were RV volumes "normalized" after surgery.

Adult↗

Postoperative prone position management of tetralogy of fallot with absent pulmonary valve syndrome.

Tetralogy of Fallot with absent pulmonary valve syndrome is commonly associated with respiratory failure both before and after surgery. This report describes our experience using prone positioning with bilateral pillows to avoid compression of the anterior chest wall after surgery. In the case here, the patient's respiratory distress was improved by this positioning. Prone position and avoiding anterior chest compression has an effect on severe respiratory distress of tetralogy of Fallot with absent pulmonary valve syndrome.

Abnormalities, Multiple↗

Prenatal diagnosis of tetralogy of Fallot with absent pulmonary valve accompanied by hydrops fetalis.

Tetralogy of Fallot with absent pulmonary valve is a rare congenital cardiac anomaly. We report on the echocardiographic features of a fetus with such an anomaly and aneurysm of the pulmonary artery associated with hydrops fetalis and polyhydramnios. The prognosis of tetralogy of Fallot with absent pulmonary valve and pulmonary aneurysm is grave, especially in a fetus with intrauterine heart failure. Serial sonographic follow-up is mandatory to detect the signs of hydrops fetalis. Early intervention and intensive treatment should be given to improve the perinatal outcome once the prenatal diagnosis is made.

Adult↗

Lung hypoplasia in congenital pulmonary valve stenosis.

The pulmonary function of ten adult patients with congenital pulmonary valvular stenosis was investigated. The patients clearly showed smaller lungs than healthy control subjects of equivalent age and height; lung elastic recoil pressure was normal at any given percentage of measured total lung capacity, indicating that postnatal parenchymal damage is not the cause of the small lungs. The lung diffusing capacity for carbon monoxide was reduced, reflecting the anatomical alterations of the pulmonary vascular bed. Finally, the maximal flow-static recoil curves showed a fixed (not dynamic) reduction of airway dimensions: the critical transmural pressure in the collapsible flow-limiting segment (Ptm') was normal, but the conductance of the S segment was lowered. These abnormalities most likely reflect inadequate development of the lung and suggest that pulmonary blood pressure may be an important determinant of lung growth in the postnatal period.

Adolescent↗

[Surgical treatment of pulmonary valve stenosis].

"Open" pulmonary valvotomy with vena cava occlusión and normothermia was first performed by Varco in 1950, but the procedure was abandoned with the arrival of extracorporeal circulation. This paper compares two groups of patients who were operated with Varco's procedure and extracorporeal circulation. Similar data of both procedures are analyzed as well as their advantages and disadvantages. It is concluded that Varco's proceduce is useful in well selected patients with no associated anomalies in which the possibility of complications of extracorporeal circulation is avoided. Also, equipment, personnel, surgical time and stay in the Intensive Care Unit and in the hospital are reduced, saving cost as much as 50%.

Body Temperature↗

Clinical and diagnostic features of pulmonary valve endocarditis in the setting of congenital cardiac malformations.

We studied the clinical and diagnostic features of 5 cases of pulmonary valve endocarditis where morphologic documentation was available. All patients had congenital cardiac disease as the predisposing factor and the infectious process involved only the pulmonary valve. None of the patients was addicted to drugs. Absence of systemic emboli, lack of pulmonary symptoms, acute course and high frequency of sterile cultures made the clinical recognition difficult and was possible only in three cases. The clinical picture of pulmonary valve endocarditis was characterised by fever, right-sided congestive cardiac failure and pulmonary incompetence. Echocardiography assumes a specific diagnostic role in such cases and cross-sectional echocardiography provides more information than M-mode echocardiography. Pulmonary valve endocarditis carries a high mortality and 3 patients died. Two patients underwent successful surgery of their underlying defect and excision of the pulmonary valve. Staphylococcus aureus was the organism grown from autopsy or surgical material in 3 cases.

Adolescent↗

Transcatheter placement of a low-profile biodegradable pulmonary valve made of small intestinal submucosa: a long-term study in a swine model.

OBJECTIVE: We sought to investigate a placement of a percutaneous low-profile prosthetic valve constructed of small intestinal submucosa in the pulmonary position in a swine model. METHODS: Twelve female farm pigs were stented at the native pulmonary valve to induce pulmonary insufficiency. Once right ventricular dilation occurred, the small intestinal submucosa valve was implanted. The pigs were followed up with transthoracic echocardiographic Doppler scanning. One animal died of heart failure before valve replacement. Animals were euthanized at 1 day, 1 month, 3 months, 6 months, and 12 months after valve implantation. RESULTS: The small intestinal submucosa pulmonary valve showed effective reversal of pulmonary regurgitation. There were no misplacements during deployment. There were no embolizations. One-year echocardiographic follow-up showed minimal regurgitation and no stenosis for a valve/vessel ratio of 0.78 or greater. Histologic examination demonstrated intensive remodeling of the small intestinal submucosal valve. Within 1 month, the surface was covered by endothelium, and fibroblasts invaded the interior. Over the following months, the small intestinal submucosal valve remodeled without apparent graft rejection. CONCLUSION: The small intestinal submucosa valve has the potential for graft longevity without the need for anticoagulation or immunosuppression. Histologic remodeling of the valve tissue provides a replacement capable of resembling a native valve that can be placed percutaneously with low-profile delivery systems.

Absorbable Implants↗

Congenital absence of the pulmonary valve.

Congenital absence of the pulmonary valve (CAPV) is a rare anomaly usually associated with ventricular septal defect or Fallot's tetralogy. CAPV may be rapidly fatal in infancy because of severe disturbance of pulmonary ventilation resulting from bronchial compression by massively dilated pulmonary arteries. Other cases may be relatively benign. Our group of five cases with CAPV ranged from the severe to one case in which there was documented closure of a patent ductus arteriosus and a ventricular septal defect, leaving the CAPV as an apparently isolated, well-tolerated lesion. Plain chest radiographs play a major part in the diagnosis of CAPV since they almost invariably give some indication od the aneurysmal dilatation of the pulmonary arteries. On angiography, dilated main pulmonary arteries are shown giving rise to branches of normal or even diminished caliber.

Angiography↗

[Results of balloon valvuloplasty in pulmonary valve stenosis].

Percutaneous balloon pulmonary valvuloplasty was performed in 17 consecutive patients, ranging in age from eleven years to 67 years (mean age: 40 +/- 17 years). The peak to peak pressure gradient was reduced by 16 to 167 mm Hg, the mean pressure gradient decreased from 99 +/- 42 to 46 +/- 22 mm Hg. In six patients there was a pressure gradient above 50 mm Hg after the procedure. Within three months it decreased due to regression of infundibular hypertrophy and ranged from 26 to 46 mm Hg after one year. There were no serious complications. One patient experienced a brief episode of syncope. Another patient developed a pulmonary incompetence which was without hemodynamic significance. Percutaneous balloon pulmonary valvuloplasty offers an alternative method for treating pulmonary stenosis not only in children but also in adults.

Adolescent↗

Tetralogy of Fallot with absent pulmonary valve and bronchial compression: treatment with endobronchial stents.

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac lesion. The lesion includes ventricular septal defect, overriding aorta, and absence of the pulmonary valve, with resultant pulmonary incompetence. It has been suggested that the pulmonary incompetence induces intrauterine dilatation of the pulmonary artery, which leads to tracheobronchial compression. One of the presenting features in infants with APVS is severe airway obstruction, which may be difficult to manage. We report an infant who benefited from bilateral endobronchial endoscopic stent placement.

Bronchial Diseases↗

Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome.

OBJECTIVE: Primary repair of tetralogy of Fallot with absent pulmonary valve syndrome has been associated with significant mortality, particularly for neonates in respiratory distress. Controversy persists regarding the method of establishing right ventricle-pulmonary artery continuity. METHODS: Anatomic and demographic parameters were evaluated for patients undergoing repair of tetralogy of Fallot with absent pulmonary valve syndrome from 1990 to 2005, as were perioperative and late postoperative parameters (airway complications, reoperation or catheter-based intervention, and mortality). RESULTS: Twenty-three patients underwent repair. Median age was 15 days (range 2-1154 days). Patients were followed up for 5.3 +/- 3.9 years. Seventeen (85%) required preoperative ventilatory assistance. One patient died within 24 hours; 1 patient died 8 months postoperatively. Four patients received valved homografts, and the remainder had valveless connections. All patients underwent reduction pulmonary arterioplasty and mobilization, unifocalization (in 3), and ventricular septal defect closure. Valveless connection recipients had a transannular hood. No patient underwent a Lecompte maneuver. Four patients underwent reoperation for conversion to valveless connection (n = 1), reduction arterioplasty (n = 1), and repair of pulmonary stenosis (n = 2). Three patients required catheter-based intervention, with balloon angioplasty (n = 3) and stent placement (n = 1); 2 now demonstrate equal quantitative lung perfusion. No patient has had significant debility from airway compromise. All patients demonstrate free pulmonary insufficiency and good biventricular function. CONCLUSIONS: We report excellent overall survival (89%) and low postoperative morbidity for neonates and infants undergoing primary repair of tetralogy of Fallot with absent pulmonary valve syndrome. Our recent experience supports the use of a valveless right ventricle-pulmonary artery connection, which, combined with catheter-based intervention, reduces the likelihood of reoperation necessitated by homograft placement.

Child, Preschool↗

Echo-Doppler features of pulmonary valve endocarditis.

Three patients with fever and pneumonitis were found to have pulmonary valve vegetations by echocardiography. Pulsed and color flow Doppler studies demonstrated mild to moderate pulmonary valve insufficiency in these patients. All patients responded to the appropriate antibiotics without requiring valve surgery. Echocardiography is of value in diagnosing pulmonary valve endocarditis and should be considered in every patient with recurrent pneumonitis.

Adult↗